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Intruduction: Testicular feminization syndrome is a disorder with the prevalence of 1 in 20000 to 64000 male births. It,s characteristics are female phenotype, male caryotype (64XY) and normal to increased testosterone and LH levels. In this article a case of testicular feminization is reported. Case: The patient was a 16 years-old woman with primary amenorrhea and without secondary sexual characteristics. The patient had normal female external genitalia, absent axillary and pubic hair, immature breast, and repaired bilateral inguinal hernia with pathology report of immature testicular tissue. FSH and LH levels were elevated. Testosteroe level was normal. Sonography and CT scan of pelvis did not show uterus and adnex. Karyotype was 46XY. Conclusion: It is recommended that due to the risk of neoplasia, the existing testes must be removed. Also hormone therapy must be done for the patient.

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Introduction: Renal cell carcinoma can be presented with extension of thrombosis to renal vein and inferior vena cava. Management of these patients consists of radical nephrectomy and thrombectomy. In this study we reviewed approach to these patients. Materials & Methods: This is a case series report. During 1379 to 1384, 148 patients diagnosed with renal cell carcinoma (RCC) limited to Gerota’s fascia (stage T3 or less) were admitted to Hasheminejad hospital. Fourteen patients had inferior vena cava (IVC) involvement. Radical nephrectomy as well as thrombectomy was performed in 8 patients. This investigation is done on these 8 patients. Results: Patients' average age was 51.4 years. Six patients were male (75%) and 2 female (25%). The most common symptoms were flank pain (75%), gross hematuria (62.5%) and structural symptoms (50%). All patients had tumors limited to Gerota’s fascia without any distant metastasis. Infrahepatic involvement of IVC was seen in 6 patients and in 2 patients thrombosis extended above the hepatic vein (infrahepatic). No perioperative mortality was seen. Postoperative complications were atelectasia in 2 patients and deep vein thrombosis in one which was treated with conservative management. Conclusion:By skillful surgical approach in patients with RCC and IVC involvement, thrombosis can be completely excised without increasing the rate of complications and longterm survival could be expected.

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Introduction: Adenosarcoma is a rare uterine tumor composed of benign epithelial and malignant stromal elements. In 20% of cases stroma contains heterologous elements (usually from stratified muscle type). Association between Tamoxifen usage (exogenous strogen) or ovarian thecoma (endogenous strogen) and occurance of this tumor has been reported. This is a case report of a uterine Adenosarcoma. Case: The patient was a 45 years old woman with history of 16 years OCP use who was admitted with a uterine mass and undergone total hysterectomy. Microscopic assessment of slides, stained by H&A, was indicator of Adenosarcoma with cartilage heterologous elements. Conclusion: Uterine Adenosarcoma is a rare tumor. Although there is a few reports of cartilage heterologous elements in uterine Adenosarcoma, this case had multiple cartilage points in stroma. Also in this case, the coexistance of this tumor with long- term OCP use was considerable. 

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Introduction: Kalleh-Pacheh is an Iranian food used in many part of Iran and many other countries such as Afghanistan, Pakistan and India. We found no information regarding the effect of this food on acute myocardial infarction. The aim of this study was to assess the effect of this food, together with diabetes and smoking on myocardial infarction in Arak district. Materials and Methods: This was a case-cohort study which was performed in Arak district. In this study the data of Arak sub-cohort was taken from Isfahan healthy heart project which was performed in 2001. Cases were myocardial infracted hospitalized patients who were resident in Arak at the time of study. We showed the effect of different exposure, on myocardial infarction by estimating relative risk and population attributable fraction and 95% confidence interval. Results: In this study 150 cases were compared with 6339 sub-cohorts. The population attributable fraction for Kalleh-Pacheh was 19% (95% CI, 6 to 30%). This measure for diabetes and smoking was 31% (95% CI, 23 to 39%) and 41% (95% CI, 31 to 49%) respectively. Conclusion: According to the results of our study smoking, diabetes and using Kalleh-Pacheh has significant effect on myocardial infarction

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Introduction: Kikuchi-Fujimoto disease or histiocytic necrotizing lymphadenitis is a rare but important etiology of cervical lymphadenopathy in young women. This disease is self limited and improves during 2-8 months. Diagnostic key is the pathology of involved lymphnode. Association of the disease with viral and autoimmune disease is not proved yet. In this article two cases of Kikuchi-Fujimoto disease are reported. Case: The first case was a 31 years old female patient with sever fever and right axillary lymphodenpathy (size: 57cm) in which one month after the disease, diagnosis was proved by pathology studies. This patient has erythematous patches in her face and nose in several times that were improved spontaneously. The second case was a 20 years old female patient with fever, chill, headache, weigh loss and cervical lymphadenopathy (size: 21cm). She received antibiotic at several times without any improvement. Finally after 5 months since the beginning of symptoms, the disease was diagnosed as Kikuchi-Fujimoto disease by surgical excision of lymhnode and pathology studies. After the surgery two small lymphnodes were appeared around the surgical site. Conclusion: In differential diagnosis of lymphadenpathy especially cervical in young women, Kikuchi-Fujimoto should be considered. Consumption of antibiotic in lymphadenopathy without clear diagnosis should be avoided. After diagnosis of Kikuchi-Fujimoto, long term work up for recurrence or the appearance of autoimmune disease symptoms is necessary.

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Introduction: Abdominal pregnancy is 1 in 10000 live births and advanced abdominal pregnancy is rare. Abdominal pregnancy is associated with high morbidity and mortality with the risk for death 7 to 8 times greater than tubal ectopic pregnancy and 90 times greater than intrauterine pregnancy. In this article a case of advanced abdominal pregnancy is reported. Case: The patient was a 35 year’s old (G4P3) woman presented with undelivered pregnancy after two years. After diagnostic procedures laparatomy was done. During the operation, fetus adhesion to the omentum was released. A 9 kg calcified fetus was delivered. Then the placenta and left adnex was removed completely. Conclusion: Regarding the high mortality rate of abdominal pregnancy, in every pregnancy specially in the precence of it’s risk factors (such as PID, history of ectopic pregnancy, history of infertilitry and using drugs to induce ovulation, IVF, prior tubal surgery and …) and also suspicious symptoms, this probability and early intervention must be considered.

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Introduction: Familial adenomatous polyposis (FAP) is a rare disease with hundreds to thousands adenomatous polyps in large intestine which inherits in the form of autosomal dominant, but 25% of patients have no positive family history. Rectorrhagia and rectal prolapsus are the most prevalent symptoms. Diagnosis is based on clinical findings and results of colon investigation, and prompt colectomy prevents carcinoma in patient. In this article a case report of familial adenomatous polyposis is presented. Case: The patient is a 17 years old teenager, a known case of familial adenomatous polyposis, who was refered with severe obstruction signs of gastrointestinal tract. The disease was diagnosied when he was 5 years old. In 6 years old he was treated by partital colectomy and in 11 years old by total colectomy and ileorectal anastomosis. The patient was investigated by endoscopy and small bowel transit. Because of many polyps in stomach, duodenum and jejunum polypectomy, gastrectomy and duodenotomy were done. Diffuse carcinoma in situ was reported in pathology. Conclusion: Rectal and colonic polyps are rarely obsereved below 10 years old, but rectorrhagia and rectal prolapsus suspects the diagnosis of FAP in children. In the case of dysplasia and carcinoma abdominal pain, anemia and weight loss will be presented. In children with suspecting FAP, screening of extracolonic manifestation and dysplasia should be considerd and elective surgery should be done.

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Introduction: Hypomagnesemia hypercalciuria nephrocalcinosis (HHNC) is a rare syndrome due to mutation defect in CLDN-16 gene. Its product is paracellin, a protein in henle loop paracellur area which acts in magnesium and calaium reabsorption. In this article a case of HHNC is reported. Case: The patient is a 3 years old boy presented with tetany, polyuria and polydipsia. Imaging study revealed medullary nephrocalcinosis. Laboratory investigations showed hypomagnesemia, hypercalciuria, hypermagnesiuria and normal arterial blood gases. In 2 years follow up, no neurological diseases, growth failure, or rickets were seen. He was treated with Hydrochlorothiazide and Potassium polycitra for prevention of nephrocalcinosis and progressive renal failure. Conclusion: HHNC is a rare syndrome presenting with hypercalciuria, hypo- magnesemia nephrocalcalcinosis, tetany, polyuria and polydypsia which progresses to end stage renal failure in 2nd or 3rd decades. Early diagnosis and some treatment modalities like Hydrochlorothiazide or polycitra-k can delay this process.

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Introduction: Wilson disease is a hereditary disorder of Copper metabolism which can present with hepatic, neurologic or psychiatric symptoms and in rare cases as a hemolytic disturbance. Free Copper can result in red blood cell damage and hemolysis which is a rare feature of the disease affecting less than 10% of patients. In this condition the liver is usually involved and liver transplantation can be life saving. This article is a case report of acute hemolytic crisis as the initial manifestation of Wilson disease. Case: An 8 years old girl was admitted in pediatric ward of Golestan hospital because of abdominal pain, icterus, anemia and tea color urine. Hepatitis or Glucose 6 Phosphate Dehydrogenase deficiency was the first diagnosis. Because of unresponsiveness to transfusion, Wilson disease was considered and the diagnosis was established with the presence of Kayser-Fleischer ring. Conclusion: Chronic or acute hemolytic anemia is a rare or unusually presentation of Wilson disease. In any child especially older than 5 years with liver disease or hemolytic anemia, Wilson disease should be considered and appropriate diagnostic tests performed

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Introduction: Sneddon syndrome is a combinaison of skin lesions (Livedo Reticularis) and acute cerebrovascular accident. The syndrome involves many organs such as kidney, heart, skin and brain. Some patients have a good prognosis when taking Aspirin and Diprydamol. In this article a case of this syndrome is reported. Case: The patient is a 40 years old woman with a history of 8 times intrauterine fetal deaths. She was admitted to the neurology ward with signs of weakness in left side of the body (hemiparesis), dysarthria, 7th central nerve paralysis, and skin lesions (Livedo Reticularis). All laboratory data such as serology and microbiology were normal. After skin biopsy from the lower limbs and rule out possible secondary etiologies, this patient was recognized with Sneddon syndrome and recieved antiplatelet treatment. Conclusion: Despite the fact that this syndrome is rare, it seems considering the probability of appearing this syndrome, especially in young patients who are suffering from acute cerebrovascular signs and skin lesions in the same time, early treatment can prevent the desease from developing to other organs.

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Background: Pyoderma gangrenosum (PG) is an uncommon, non-infectious, neutrophilic, ulcerative disease with typically starts with pustules which rapidly evolve to painful ulcers with undermined violaceous borders. The diagnosis of PG is based on clinical features and requires exculusion of other conditions that produce ulcerations .PG is associated with a variety of systemic diseases that the most commonly of them is include inflammatory bowel diseases, leukaemia, myeloma, monoclonal gammopathies, chronic active hepatitis, systemic lupus erythmatous and arthritis. Two cases of PG with haemolytic anemia have been reported and this patient is third one. Case: patien was a 19-year –old girl, with haemolytic anaemia from 15 years ago. Who was reffered with an ulcer, 2×3 cm in diameter, on her lower right leg with violaceous border and on her lower left leg with painful haemorrhagic bulla 4×6 cm in diameter. Conclusion: Because, PG in many cases is associated with an underlying disease in these patients, search for finding possible underlying diseases is necessary

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 Abstract Introduction: Demodex is a genous from family of Demodicidae, order of Prostigmata, Demodex is a genus of tiny parasitic mites that live in or near hair follicles of mammals. Two species living on humans have been identified: Demodex folliculorum that live in or near hair follicles of mammals and Demodex brevis. This diseas in women ocure more than men Case: The patient was a lady with 20 years old, who lives in a village urban of Arak. She was referred with sever itch and hyperkeratosis in cheek to dermatologist. She has referred to clinical pathology laboratory. Several slides have provided from skin of cheek.The density of Demodex was seen under the microscopic study in this patient. Conclusion: The study suggests that patients have referred with itch and hyperkeratosis in head and face may be suffered from demodex infection. Thus it is suggested that demodex diagnosis add to standard diagnosis paradigm.

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Background: Hydatid cyst, a parasite disease, can involve liver more than other organs. The most serious complication of the cyst is rupture which may spread out to biliary duct, hollow organ, or abdominal cavity directly. Elective treatment is emergent surgery. Case: A 28 rural woman referring with abdominal pain, nausea and vomiting due to car accident. Abnormal findings consisted of: abdominal distention, reduction of bowel sounds, tenderness and generalized guarding and hive lesions in the anterior chest wall, upper abdomen, and both forearms. In sonography, a lot of free liquid in abdominal and pelvic cavity, and three hetroechoic masses in the right lobe of the liver were reported. Four days after abdominal surgery the patient was discharged with Albendazole order. Conclusion: Intra-abdominal rupture of hydatid cyst is the most serious that due to trauma complication and elective treatment is emergency surgery.

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Introduction: Cecal volvulus is an emergency surgical acute abdomen. In this condition cecum, ileum terminal and ascending colon becomes twisted on its mesenteric anticlock wise direction rotation ranging 180-360 degrees. The cecal volvulus is caused by a lack of fixation of cecum to retroperitoneum. The usual presentation consists of severe colicky abdominal pain with asymmetric abdominal distension. In this article two cases of cecal volvulus are presented. Cases: The first case was a 74 year old female which presented with sudden abdominal pain & asymmetrical severe distension following 3 days constipation. After Plain abdominal x-ray and abdominal CT scan, right hemicolectomy was performed. The second case was a 70 year old female who following 4 days constipation was presented with abdominal pain and distension. Supine and erect abdominal x-ray showed colon hyperaeration and multiple air-fluid levels. The gangrenous colon was resected and colostomy was done. Conclusion: Despite other causes of intestinal obstruction or sigmoid volvulus which can sometimes be treated medically, the treatment of cecal volvolus is always surgery and consisted of cecum and ascending colon resection.

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Background: Brucellosis, a zoonotic disease with worldwide spread, is a common disease in Iran and is endemic in Markazi Province. Brucella osteomyelitis, on the other hand, is a rare disease. Here we reported a case of brucella osteomyelitis of the tibia following trauma. Case: The patient was a 22 year old man referring with chronic draining tibia ulcer. He had a history of trauma of the tibia, dating back to 3 years ago, for which local prosthesis had been prescribed. Later on, the prosthesis had been removed due to infection. Staining of the tissue samples indicated the presence of gram-negative coccobacill and brucella growth in the culture of excretions. Conclusion: This case was reported because of rare presentation of brucella and the educational implications of the patient with respect to the possibility of brucella development in chronic ulcers or prosthesis. Although uncommon, brucellosis should be regarded as a potential cause of osteomyelitis in patients with a record of physical contact with animals.

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Background: Spontaneous rupture of tendon is a rare disease that often occurs following an underlying disease or drug usage. However, the association of this condition with primary hyperparathyroidism has rarely been reported. Case: In a case-study, two cases of spontaneous tendon rupture associated with primary hyperparathyroidism are reviewed. The first one is a middle-aged woman that due to bilateral patellar tendon rupture is operated for secondary repair and augmentation of tendons. The second one is a middle-aged man with unilateral triceps brachii tendon rupture who has been operated for primary tendon repair. Conclusion: Since paramount force is needed for tendon rupture, in spontaneous tendon rupture cases, patient evaluation for detecting underlying diseases and drug usage is necessary. In cases that the history of an underlying disease is not indicated, evaluation of serum level concentrations of Ca, P, ALP, and PTH is recommended for contraindication of primary hyperparathyroidism.

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Background: Central giant cell granuloma (CGCG), also known as giant cell reparative granuloma, is a non-neoplastic proliferative lesion with unknown etiology which commonly occurs in the right side of the mandible. This lesion presents a wide variety of radiological and clinical manifestations that may lead to misdiagnosis. In many situations, CGCG is diagnosed through histopathological examinations. Case: In this study, a case of CGCG with irregular clinical and radiological aspect arising from the anterior, left side of the maxilla was reported. Conclusion: In differential diagnosis of swellings arising from the anterior portion of the maxilla, the possibility of CGCG should be considered. Also, histopathological examinations are of great importance in the diagnosis of CGCG.

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Background: Mycobacterium marinum is an atypical mycobacterium that causes a type of skin infection known as fish tank granuloma affecting people who are exposed to aquatic environments and manifests with erythematous, papulonodular or plaque-like lesions of the extremities. Case: A 15 year-old woman working in a tropical aquarium in Khomein referred with a non-tender nodule on the dorsum of fourth finger of left hand for 4 months. There were no specific findings in physical examination. Routine laboratory tests were normal. Skin biopsy was obtained which revealed suppurative granulomas, but staining of PAS and Ziel-Nelson were negative. The patient was treated with doxycycline 100 mg P.O. BID for 2 months with complete improvement of the lesions. Conclusion: In patients with a history of work in aquatic environments who manifest erythematous, papulonodular, or plaque-like lesions of the extremities should be checked in terms of fish tank granuloma and infection with mycobacterium marinum and skin biopsy and culture should be done for lesions.

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Background: Transitional cell carcinoma is the most common bladder tumor that can be treated by surgery, BCG instillation, chemotherapy, and radiotherapy. This study deals with urethral squamous cell carcinoma (SCC) in a patient with history of transurethral resection (TUR-T) of bladder transitional cell carcinoma treated by BCG instillation. Case: A 68-year-old man was admitted with gross hematuria, lateral prostatic lobe kissing, and a pedunculated 2×3mm mass in bladder in cystoscopy. He had undergone TUR-T and BCG instillation and had been followed regularly every 3 months with cystoscopy. In the follow up, 9 months later, several pedunculated tumors in penile urethra that were of SCC type were indicated in pathological analysis. Conclusion:: Since membranous urethra is the most common site and TCC is the most common type of urethral cancers in this site, SCC in penile urethra was unexpected. This could be because of chronic inflammation due to treatment and cystoscopic follow ups.

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Background: Localization of ectopic parathyroid glands is one of the most sophisticated challenges in endocrine surgery and there is considerable controversy regarding the suitable approach to localize ectopic parathyroid glands and indications for using different modalities. The failure of the first operation leads to pain, scar, and financial burden of the following operation. Case: The patient was a 39-year-old woman hospitalized with hematuria as the main complaint and noticing the presentation of hypercalcemia in the initial tests underwent more thorough examination. Ultrasonography and 99mTc sestamibi scan helped us to localize the ectopic parathyroid gland in an anterior mediastinum and treat the patient in a one stage operation. Conclusion: Paying careful attention to the symptoms and signs, considering all differential diagnoses, and using appropriate diagnostic methods can be helpful in the diagnosis of rare syndromes.